Patients with Darier disease have an increased risk of keratinocyte carcinoma: a Swedish registry-based nationwide cohort study.

Autor: Inci R; Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden. rahime.inci@gu.se.; Region Västra Götaland, Department of Dermatology and Venereology, Sahlgrenska University Hospital, Gothenburg, Sweden. rahime.inci@gu.se., Gillstedt M; Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden.; Region Västra Götaland, Department of Dermatology and Venereology, Sahlgrenska University Hospital, Gothenburg, Sweden., Kallionpää RA; Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden.; Cancer Research Unit and FICAN West Cancer Centre, Institute of Biomedicine, University of Turku, Turku, Finland., Peltonen S; Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden.; Region Västra Götaland, Department of Dermatology and Venereology, Sahlgrenska University Hospital, Gothenburg, Sweden.; Department of Dermatology and Allergology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland., Polesie S; Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden.; Region Västra Götaland, Department of Dermatology and Venereology, Sahlgrenska University Hospital, Gothenburg, Sweden.
Jazyk: angličtina
Zdroj: Orphanet journal of rare diseases [Orphanet J Rare Dis] 2024 Dec 16; Vol. 19 (1), pp. 463. Date of Electronic Publication: 2024 Dec 16.
DOI: 10.1186/s13023-024-03497-z
Abstrakt: Background: Darier disease is a genodermatosis which manifests as hyperkeratotic papules and superficial erosions mainly in seborrheic skin areas. This retrospective registry-based cohort study aimed to estimate the association between Darier disease and skin cancer.
Results: Patients diagnosed with Darier disease were identified from the patient registry of Sahlgrenska University Hospital (Gothenburg, Sweden) in 2016-2020. The local cohort included 13 patients. Verification of Darier disease diagnosis in the National Patient Registry showed positive predictive value of more than 90%. National Patient Registry was searched for Darier disease in 2001-2020, Swedish Cancer Registry for cancers and Prescribed Drug Register for medications. The national cohort included 770 patients and tenfold matched control cohort. Patients with Darier disease had an increased relative risk of keratinocyte carcinoma (basal cell carcinoma and cutaneous squamous cell carcinoma combined) (hazard ratio [HR], 1.6, 95% confidence interval [CI], 1.0-2.5, P = 0.036). The risk increase was significant for basal cell carcinoma (HR, 1.8, 95% CI, 1.1-2.9, P = 0.012), whereas there was a trend for cutaneous squamous cell carcinoma, (HR, 1.9, 95% CI, 0.9-4.1, P = 0.086) and cutaneous melanoma (HR, 2.4, 95% CI, 0.9-6.2, P = 0.083). Standardized incidence ratio for keratinocyte cancers was 2.9 (95% CI, 2.4-3.3. The results were consistent in two subgroup analyses adjusting for use of retinoid and/or immunosuppressive drugs.
Conclusion: Patients with Darier disease have an increased risk of skin cancer, particularly keratinocyte carcinoma. This risk was consistent even when known risk modifiers for keratinocyte carcinoma were excluded in sensitivity analyses. The results support previously proposed molecular links between Darier disease and skin cancer, but further investigations are needed. Additional studies are also required to develop clinical management recommendations for Darier disease. In the meantime, dermatologists should be aware of the cancer risk in these patients and remain vigilant, as detecting cancer can be challenging in hyperkeratotic and/or eroded skin.
Competing Interests: Declarations. Ethics approval and consent to participate: The study was carried out with the approval of the Swedish Ethical Review Authority (approval number: 2020–06202) and Sahlgrenska University Hospital (SUH). Consent for publication: The patients have provided written informed consent for the publication of their photos in this publication. Competing interests: The authors declare that they have no conflict of interest.
(© 2024. The Author(s).)
Databáze: MEDLINE
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